The triad of ataxia, areflexia and ophthalmoplegia was first described as a variant of the Guillain-Barre syndrome in 1932 by Collier. In 1956, Miller Fisher reported three patients with ataxia, areflexia, and ophthalmoplegia as a separate entity. Since then, 223 cases of Miller Fisher syndrome have been published. The male/female ratio is 2:1 with a mean age of 43.6 years at the onset of the disease. A viral infection preceded the neurological symptoms in 71.8% of cases with an average symptom-free interval of 10 days. First symptoms were diplopia (38.6%) or ataxia (20.6%). An areflexia was present in 81.6% of cases. Cranial nerves other than the oculomotor nerves were involved in 127 cases (56.9%): cranial nerves 7 (45.7%), 9 and 10 (39.9%), and 12 (13%) were involved, in 53 cases a tetraparesis occurred. An elevated protein value was present in 134 patients (64.4%); cerebrospinal fluid findings were normal in 56 panents. Eighteen patients showed a mild pleocytosis. Besides the cerebellar type of ataxia, initial disturbances of consciousness (n = 8), supranuclear oculomotor signs (n = 22), and pathology findings in electroencephalography (n = 38), computed tomography (n = 8), and magnetic resonance imaging (n = 2) were reported as evidence for a central nervous system involvement in the Miller Fisher syndrome. The prognosis of Miller Fisher syndrome was good recovery occurred after a mean time period of 10.1 weeks. Residual symptoms were present in 74 cases (33.2%), and a recidivism of the Miller Fisher syndrome was reported in seven patients. Eight patients died. Of six patients with autopsy findings, four exhibited central nervous system lesions. The Miller Fisher Syndrome is apparently a variant of the Guillain-Barre syndrome with frequent central nervous system involvement and a benign outcome in the majority of cases.
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